儿童癌症幸存者成年后的慢性疾病

由于治疗方法的进步,近80%的儿童和青少年癌症患者能够长期生存。在美国,约有270000例儿童癌症的幸存者,即每640名20至39岁成年人中就有一名幸存者。大量的幸存者有利于儿童癌症治疗后长期健康结果的研究。现在可以明确的是,化疗和放疗所致的儿童各器官系统损害在临床上可能潜伏多年。为了全面了解治疗儿童癌症而继发的健康问题,重要的是衡量三项长期结果:健康状况、死亡率和患病率。这三项中,关于前两项已有相当好的研究报道。在一项对20227例癌症5年生存者的回顾性分析中,Mertens等发现以下原因导致的超额死亡率具有统计学意义:继发癌症(…     

Mutations in Emery-Dreifuss muscular dystrophy and their effects on emerin protein expression

Seventeen families with Emery-Dreifuss muscular dystrophy (EDMD) have been studied both by DNA sequencing and by emerin protein expression. Fourteen had mutations in the X-linked emerin gene, while three showed evidence of autosomal inheritance. Twelve of the 14 emerin mutations caused early termination of translation. An in-frame deletion of six amino acids from the C-terminal transmembrane helix caused almost complete absence of emerin from muscle with no localization to the nuclear membrane, although mRNA levels were normal. This shows that mutant emerin proteins are unstable if they are unable to integrate into a membrane. A 22 bp deletion in the promoter region was expected to result in reduced emerin production, but normal amounts of emerin of normal size were found in leucocytes and lymphoblastoid cell lines. This shows that DNA analysis is necessary to exclude emerin mutations in suspected X-linked EDMD. Emerin levels in female carriers often deviated from the expected 50% and this was due, in at least two families, to skewed emerin mRNA expression from the normal and mutated alleles. In one family with a novel deletion of the last three exons of the emerin gene, a carrier had a cardiomyopathy and very low emerin levels (<5% of normal) due to skewed X-inactivation. In the three autosomal cases of EDMD, emerin was normal on western blots of blood cells, which suggests that autosomal EDMD is not caused by indirect reduction of emerin levels.      

Catch-up growth up to ten years of age in children born very preterm or with very low birth weight

Background  

Improved survival due to advances in neonatal care has brought issues such as postnatal growth and development more to the focus of our attention. Most studies report stunting in children born very preterm and/or small for gestational age. In this article we study the growth pattern of these children and aim to identify factors associated with postnatal catch-up growth.     

腹腔镜外科和妇科联合手术的临床应用

目的：探讨腹腔镜技术在外科和妇科疾病治疗中联合应用的临床价值。　
方法：总结近7年来施行腹腔镜联合手术治疗妇、外科疾病229例的临床资料,其中腹腔镜胆囊切除术(laparoscopic cholecystectomy,LC)联合输卵管造口术5例,LC联合卵巢囊肿剥除术28例,LC联合子宫肌瘤剔除术25例,LC联合子宫次全切除术39例,LC联合子宫全切除术26例,LC联合子宫内膜异位症手术6例;腹腔镜阑尾切除术(laparoscopicappendectomy,LA)联合输卵管手术38例,LA联合卵巢囊肿切除术32例,LA联合子宫全切除术或子宫次全切除术24例,腹腔镜肝囊肿开窗引流术联合卵巢囊肿切除术6例。
结果：229例妇、外科联合腹腔镜手术均获成功,无中转开腹手术。手术时间40~220 min,平均120 min;住院1~6 d,平均3.4 d。仅1例术后10 d阴道残端出血非手术疗法治愈。175例随访3~24个(平均19.5)月,1例术后2个月发现阴道残端息肉,经手术切除治愈。　
结论：严格掌握联合手术指征,充分术前准备,多科室良好配合,腹腔镜联合手术能够有效地同时处理外科和妇科并存疾病,在基层医院具有良好的应用前景。     

日本血吸虫SjCa8基因的原核表达及其免疫学特征分析

目的表达和纯化日本血吸虫钙结合蛋白(SjCa8),研究其免疫学特征。方法重组质粒pET32a ( )-SjCa8经诱导表达后,获取纯化蛋白SjCa8,利用ELISA、Western-blot和动物保护性实验检测SjCa8的免疫学特征。结果成功获取了纯化蛋白SjCa8,该蛋白能免疫识别血吸虫感染小鼠血清和血吸虫病患者血清。SjCa8免疫Balb/c小鼠可获得较高滴度的特异性抗体,诱导宿主产生35．31%的减虫率和35．68%的减卵率。结论纯化蛋白SjCa8具有良好的免疫原性和免疫反应性,具有作为血吸虫病免疫诊断和疫苗候选分子的研究价值。     

几丁糖与人工骨联合应用预防椎板切除术后硬膜外粘连的实验研究

目的:观察椎板切除术后用几丁糖(CHT)的同时在椎板缺损处植入磷酸三钙人工骨(TCP)预防硬膜外瘢痕粘连的效果。方法:40只纯种日本大耳白兔随机分成A、B、C、D四组,制作L4椎板损伤模型。A组硬膜外涂布生理盐水,B组硬膜外涂布几丁糖,C组硬膜外覆盖人工骨,D组硬膜外涂布几丁糖的同时加人工骨覆盖。术后12周对椎板切除部位进行大体观察、组织学观察及生化检查,比较各组间瘢痕形成和粘连情况。结果:B、C、D组的改良Rydell-Balazs粘连韧度评分、胶原含量及改良Nussvaum组织学评分均优于A组(P<0.01),D组优于B组与C组(P<0.05),B组与C组无显著性差异(P>0.05)。结论:联合应用几丁糖和人工骨能有效预防硬膜外瘢痕粘连的形成,比单独应用几丁糖和人工骨预防效果好。     

Regulation of intercellular adhesion molecule-1 gene expression involves multiple mRNA stabilization mechanisms: effects of interferon- gamma and phorbol myristate acetate

Although the intercellular adhesion molecule-1 (ICAM-1) is constitutively expressed at a low level on a subpopulation of hematopoietic cells, on vascular endothelium, on fibroblasts, and on certain epithelial cells, it is dramatically increased at sites of inflammation. Interferon-gamma (IFN-gamma) and phorbol myristate acetate (PMA) are known to increase the expression of ICAM-1 on many cell types. Because both human and murine ICAM-1 mRNAs contain putative destabilizing AUUUA sequences in their 3' untranslated regions (UTRs), we examined the role of mRNA stability in the regulation of ICAM-1 gene expression. The treatment of the murine monocytic cell line P388D1, which constitutively expresses ICAM-1 mRNA at a low level, with IFN- gamma or PMA rapidly enhanced the level of ICAM-1 mRNA and dramatically prolonged its half-life. To determine whether the putative destabilizing sequences are responsible for this effect of IFN-gamma and PMA, fibroblast L cells were transfected with either the full- length ICAM-1 cDNA or a truncated form (ICAM-1 delta 3) lacking the putative destabilizing AUUUA sequences. Although ICAM-1 delta 3 mRNA was more stable than the full-length ICAM-1 mRNA, IFN-gamma treatment induced the accumulation of both mRNA species and prolongation of their half-lives. The transplantation of the ICAM-1 delta 3' UTR into a stable ICAM-2 mRNA rendered it unstable, and it was unresponsive to IFN- gamma. Therefore, the treatment with IFN-gamma stabilizes the otherwise labile ICAM-1 mRNA, but the IFN-gamma-responsive sequence may at least in part reside within the protein coding region. PMA also upregulated ICAM-1 gene expression by mRNA stabilization. However, unlike IFN- gamma, PMA treatment only increased the level of the full-length, but not of the truncated, ICAM-1 mRNA. This shows that the PMA-responsive element is located within the 3'UTR. Furthermore, the effect of PMA on ICAM-1 delta 3 mRNA was recovered by ligating multiple AUUUA sequences derived from a heterologous gene fragment. The stability of this chimeric mRNA and the full-length ICAM-1 mRNA was markedly increased by PMA treatment, indicating that the AUUUA multimers in the 3'UTR are important in the PMA-induced upregulation of ICAM-1 mRNA.     

变应性鼻炎脱敏治疗的效果观察

目的观察变应性鼻炎（AR）脱敏治疗的效果，探讨影响变应性鼻炎脱敏治疗效果的相关因素。方法对185例AR患者通过皮肤试验，选择敏感变应原浸液，按递增剂量进行常规皮下脱敏治疗。根据患者治疗前后的症状和体征得分情况，分为显效、有效和无效。选择性别、年龄、病程、病情、伴慢性鼻窦炎、伴哮喘、脱敏治疗时间、皮试反应等8个影响变应性鼻炎脱敏治疗效果的可能因素进行多元回归分析，筛查影响脱敏治疗效果的因素。结果总有效率74．6％，其中显效22．9％，有效51．7％。影响脱敏治疗效果的主要因素是治疗时间，即时间越长，效果越好（P〈0．01）；其次是年龄因素，年龄越小，效果越好（P〈0．05）；伴慢性鼻窦炎和伴哮喘也可能影响脱敏治疗效果（P值分别为0．072和0．069）。结论变应性鼻炎脱敏治疗是有效的，脱敏治疗时间越长、年龄越小，效果越好。